Maintenance of X chromosome inactivation after T cell activation requires NF-κB signaling

Sci Immunol. 2024 Oct 4;9(100):eado0398. doi: 10.1126/sciimmunol.ado0398. Epub 2024 Oct 4.

Abstract

X chromosome inactivation (XCI) balances X-linked gene dosage between sexes. Unstimulated T cells lack cytological enrichment of X-inactive specific transcript (Xist) RNA and heterochromatic modifications on the inactive X chromosome (Xi), which are involved in maintenance of XCI, and these modifications return to the Xi after stimulation. Here, we examined allele-specific gene expression and epigenomic profiles of the Xi in T cells. We found that the Xi in unstimulated T cells is largely dosage compensated and enriched with the repressive H3K27me3 modification but not the H2AK119-ubiquitin (Ub) mark. Upon T cell stimulation mediated by both CD3 and CD28, the Xi accumulated H2AK119-Ub at gene regions of previous H3K27me3 enrichment. T cell receptor (TCR) engagement, specifically NF-κB signaling downstream of the TCR, was required for Xist RNA localization to the Xi. Disruption of NF-κB signaling in mouse and human T cells using genetic deletion, chemical inhibitors, and patients with immunodeficiencies prevented Xist/XIST RNA accumulation at the Xi and altered X-linked gene expression. Our findings reveal a previously undescribed connection between NF-κB signaling pathways, which affects XCI maintenance in T cells in females.

MeSH terms

  • Animals
  • Female
  • Humans
  • Lymphocyte Activation* / immunology
  • Male
  • Mice
  • Mice, Inbred C57BL
  • NF-kappa B* / immunology
  • NF-kappa B* / metabolism
  • RNA, Long Noncoding / genetics
  • RNA, Long Noncoding / immunology
  • Signal Transduction* / immunology
  • T-Lymphocytes* / immunology
  • X Chromosome Inactivation* / immunology

Substances

  • NF-kappa B
  • XIST non-coding RNA
  • RNA, Long Noncoding